title: Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII: a novel form of acquired factor XIII deficiency.
abstract: Acquired factor XIII (FXIII) deficiency due to autoantibody against FXIII is a very rare severe hemorrhagic diathesis. Antibodies directed against the A subunit of FXIII, which interfere with different functions of FXIII, have been described. Here, for the first time, we report an autoantibody against the B subunit of FXIII (FXIII-B) that caused life-threatening bleeding in a patient with systemic lupus erythematosus. FXIII activity, FXIII-A(2)B(2) complex, and individual FXIII subunits were undetectable in the plasma, whereas platelet FXIII activity and antigen were normal. Neither FXIII activation nor its activity was inhibited by the antibody, which bound to structural epitope(s) on both free and complexed FXIII-B. The autoantibody highly accelerated the elimination of FXIII from the circulation. FXIII supplementation combined with immunosuppressive therapy, plasmapheresis, immunoglobulin, and anti-CD20 treatment resulted in the patient's recovery. FXIII levels returned to around 20% at discharge and after gradual increase the levels stabilized above 50%.
authors.affiliation: Clinical Research Center, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary.
authors.forename: Eva
authors.initials: E
authors.lastname: Ajzner
authors.forename: Agota
authors.initials: A
authors.lastname: Schlammadinger
authors.forename: Adrienne
authors.initials: A
authors.lastname: Kerényi
authors.forename: Zsuzsanna
authors.initials: Z
authors.lastname: Bereczky
authors.forename: Eva
authors.initials: E
authors.lastname: Katona
authors.forename: Gizella
authors.initials: G
authors.lastname: Haramura
authors.forename: Zoltán
authors.initials: Z
authors.lastname: Boda
authors.forename: László
authors.initials: L
authors.lastname: Muszbek
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology*; D015153:Blotting, Western; D004797:Enzyme-Linked Immunosorbent Assay; D005176:Factor XIII / Q000032:analysis / Q000276:immunology*; D005177:Factor XIII Deficiency / Q000276:immunology* / Q000628:therapy; D005260:Female; D006470:Hemorrhage / Q000276:immunology*; D006801:Humans; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007676:Kidney Failure, Chronic / Q000209:etiology; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D010956:Plasmapheresis; D006435:Renal Dialysis; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D001324:Autoantigens; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D005176:Factor XIII; C054380:factor XIIIb